Juvenile acanthosis nigricans in twins with neurological changes including spastic paraparesis.
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Acanthosis Nigricans↗
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[SPASTIC PARAPARESIS AS AN INITIAL CLINICAL MANIFESTATION OF DIABETES].
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Diabetes Mellitus↗
Masked deficit of vitamin B12 in the patient with heterozygous beta-thalassemia and spastic paraparesis.
The spinal cord, brain, optic nerves and peripheral nerves may be affected by vitamin B12 (cobalamin) deficiency. Deficiency of vitamin B12 also causes megaloblastic anaemia, meaning that the red blood cells are usually larger than normal. In this paper we report a 16-year old girl who was referred to us for the evaluation of mild paraparesis and paresthesias marked by tingling "pins and needles" feelings and general weakness. The patient, her parents and sisters were on a strict vegan diet, which made us believe that vitamin B12 deficiency may be the possible cause of the neurologic clinical manifestations. The serum level of vitamin B12 was low, but there was no macrocytosis in the routine blood examination. The electrophoresis of haemoglobin was pathologic, there was 3.7% of HbA2 and 11.6% of HbF (heterozygous form of beta-thalassaemia). When megaloblastic anaemia occurs in combination with a condition that gives rise to microcytic anaemia, many megaloblastic features may be masked. Instead of being macrocytic, the anaemia could be normocytic or even microcytic. Vitamin B12 deficiency is a diagnosis that must not be overlooked. This case report turns the light on the fact that increased MCV is a hallmark in vitamin B12 deficiency, but it is not an obligatory sign.
Adolescent↗
[Anestesia in a man with hereditary spastic paraparesis].
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Anesthesia↗
[Tropical spastic paraparesis due to HTLV-I. 4 case reports and literature review].
We describe the clinical, radiological, neurophysiological and biological characteristics of 4 patients suffering from tropical spastic paraparesis due to a HTLV-I infection. Patient 1 immigrated in Europe at the age of 16 from Caribbean islands. Patient 2 was a White male Caucasian who lived during several years with a prostitute also native of Caribbean islands. Patient 3 was a Black male Zaïrian and Patient 4 was a female of mixed race who lived many years in Zaäire and in Senegal. All the 4 patients presented with walking difficulties, a spastic paraparesis, urinary disturbances and often, severe constipation. The diagnosis was based on a positive ELISA test for HTLV-I antibodies, confirmed in 2 cases each by Western blots and detection of the viral genome by the Polymerase Chain Reaction. In all cases, CSF pleocytosis and CSF-restricted oligoclonal IgG bands were observed. MRI of the spinal cord showed a global atrophy in 3 cases. Cerebral MRI revealed unusual extensive, progressive and symmetric lesions of the white matter in Patient 1, and some patchy periventricular lesions in Patient 4; this examination was normal in the two other cases. Sensory evoked potentials indicated abnormalities of central conduction velocities at the level of the posterior columns. The pathogenesis of tropical spastic paraparesis is briefly discussed.
Aged↗
Tumor necrosis factor alpha expression in the spinal cord of human T-cell lymphotrophic virus type I associated myelopathy/tropical spastic paraparesis patients.
HTLV-I Associated Myelopathy Tropical Spastic Paraparesis (HAM/TSP) is a chronic degenerative disease mainly affecting the spinal cord. The pathogenesis of HAM/TSP is unknown, but is thought to involve immunopathogenic mechanisms. Several reports have detected inflammatory cytokines such as tumor necrosis factor alpha (TNF alpha) in HAM/TSP patients. In this study, we used in situ hybridization (ISH) to examine the expression of TNF alpha RNA in spinal cord autopsy specimens from three chronic HAM/TSP patients with long-term disease (10-20 years duration). ISH identified many TNF alpha-expressing cells throughout all three patient's spinal cord tissues. Two patient's spinal cord tissue showed inflammatory cells, however double labeling by ISH for TNF alpha RNA with immunohisto-chemistry for CD45RO (a marker for memory T-cells) or CD-68 (a marker for microglia/macrophages) did not colocalize TNF alpha RNA with either CD45RO or CD-68 positive cells. Therefore, TNF alpha is expressed in the spinal cord of chronic HAM/TSP patients compared to normal controls and TNF alpha-expressing cells do not appear to be memory T-cells, microglia or macrophages.
Antigens, CD↗
Investigation of mitochondrial function in hereditary spastic paraparesis.
Following the association of hereditary spastic paraparesis (HSP) with mutation in the paraplegin gene (SPG7) and mitochondrial dysfunction, we wished to investigate whether mitochondrial dysfunction might be associated with other forms of HSP. Five cases of HSP caused by mutation in the spastin gene (SPG4) and nine cases with HSP with mutation in the spastin and paraplegin genes excluded (non-SPG4/SPG7), were investigated for mitochondrial dysfunction. Muscle tissue from the HSP groups and a control group was analysed histochemically and spectrophotometrically for mitochondrial dysfunction. A significant decrease in mitochondrial respiratory chain complexes I and IV was demonstrated in the non-SPG4/SPG7 group. No abnormality was detected in the SPG4 group. We therefore conclude that there is evidence for mitochondrial dysfunction in non-SPG4/SPG7 HSP. There is no evidence for mitochondrial dysfunction in the pathogenesis of spastin-related HSP.
ATPases Associated with Diverse Cellular Activitie↗
[HTLV-I seronegative idiopathic progressive spastic paraparesis: clinical and neurophysiological study of the sensory features].
BACKGROUND: HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a defined entity. However, there are many patients not well characterized with a similar clinical picture who are HTLV-I seronegative. OBJECTIVE: Clinical and neurophysiological description of patients with HTLV-I seronegative idiopathic paraparesis. PATIENTS AND METHODS: Seventeen patients (4 women and 13 men aged 24-67 years, average 52.3) were evaluated including clinical assessment, vibratory sensory analysis, quantitative somatosensory thermotest (QST), somatosensory evoked potentials (SSEPs), electromyography (EMG) and motor and sensory nerve conductions. RESULTS: In addition to the spastic paraparesis, 3 (17.6%) patients had pseudobulbar symptoms. Ten (58.8%) patients had a spastic gait but could walk unaided, 6 (35.2%) needed support and 1 patient could not walk. Bladder dysfunction was found in 10 (58.8) patients and sensory symptoms in 7 (41.1%). There was mild distal impairment of vibration and position sense, distal tactile and pinprick hypoesthesia in 4 (23.4%) patients. Tibial SSEPs were abnormal in 11 (64.7%). Nerve conduction studies and EMG were normal. QST showed cold hypoesthesia in 14 (82.4%) patients. Warm sensation and beat pain appeared unimpaired. CONCLUSIONS: All sensory abnormalities found were restricted to sensations carried by myelinated (A beta and A delta) channels. Sensory and motor abnormalities are similar to HAM/TSP patients suggesting a common pathogenesis.
Adult↗
Intrathecal synthesis of IgG antibodies to HTLV-I supports an etiological role for HTLV-I in tropical spastic paraparesis.
High titers of antibody to human T-lymphotropic virus type I (HTLV-I) have been reported in the sera and cerebrospinal fluids of patients with tropical spastic paraparesis. By means of agarose isoelectric focusing and selective immunoblotting, we demonstrated oligoclonal bands of immunoglobulin G antibodies to HTLV-I in the serum and cerebrospinal fluid of patients with tropical spastic paraparesis. Such cerebrospinal fluid-specific immunoglobulin bands indicate intrathecal synthesis of specific antibodies to HTLV-I. These findings mimic the antibody response to measles virus in subacute sclerosing panencephalitis and support an etiological role for HTLV-I in the pathogenesis of tropical spastic paraparesis.
Antibodies, Anti-Idiotypic↗
Spastic paraparesis of obscure origin. A case-control study of HTLV-I positive and negative patients from Rio de Janeiro, Brazil.
In order to find clinical findings that could significantly discriminate between HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and HTLV-I negative spastic paraparesis of obscure origin (SPOneg) prior to serological testing, and to find risk factors significantly associated with HAM/TSP we devised a case-control study. Sixty consecutive SPO patients were studied without previous knowledge of their HTLV-I serological status. Thirty-four (56.7%) turned out to be HTLV-I positive and 26 (43.3%) HTLV-I negative. HTLV-I infected patients tended to have more commonly motor and bladder disturbances at the beginning of their illness and a disease that was still in progression at the time of the examination. Bladder dysfunction, constipation and penile impotence, and more widespread pyramidal signs, were also more frequent during the whole course of their illness. Likewise, an increased intrathecal synthesis of IgG was found more often in the HTLV-I positive group. The only risk factor for HTLV-I infection significantly associated to HAM/TSP was a prior history of sexually transmitted diseases. These results suggest that, at least in RJ, HAM/TSP might be mainly sexually acquired.
Adolescent↗
[Tropical spastic paraparesis (HAM/TSP) in different regions of the world. Subclinical and geographic differences in neurophysiologic studies].
Tropical spastic paraparesis is the same clinical entity all over the world. It has been suggested that some clinical presentation differences are due to not well defined environmental factors that act along with the virus, modifying the start and progression of the disease. We analyzed 25 publications on the disease coming from different parts of the world, aiming to unravel regional differences. In these reports, we found different degrees of subclinical abnormalities in visual and somatosensory evoked potentials, nerve conduction studies, superior and inferior limb F wave and electromyography. It is concluded that genetic, environmental and even cultural factors may influence the clinical presentation of tropical spastic paraparesis and explain the detected differences in the reports analyzed in this study.
Electromyography↗
A clinical neurophysiologic study of tropical spastic paraparesis.
During a field study in the Seychelles Islands, 19 patients with tropical spastic paraparesis (TSP) were evaluated electrophysiologically. Methods of assessment included motor and sensory nerve conduction studies, electromyography, and analysis of the somatosensory evoked potentials after stimulation of the tibial and median nerves. The results showed that the most prominent feature of the disease, a spastic paraparesis, is accompanied by subclinical involvement of spinal sensory pathways and a comparatively minor peripheral sensorimotor polyneuropathy.
Electromyography↗
Clinical and demographic features of HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) in Rio de Janeiro, Brazil.
In Rio de Janeiro (RJ) most cases of paraparesis of obscure origin are associated with the human T-cell lymphotropic virus type I (HTLV-I). Thirty-four consecutive patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) from RJ were evaluated. Most patients came from low socio-economic levels. There was no difference in terms of gender. The main affected racial group was white. A history of sexually transmitted diseases was a major risk factor for HAM/TSP and a positive serology for syphilis was found in 26.5% of the patients. The major clinical findings were of a spastic paraparesis with generalized brisk tendon jerks and bilateral Babinki's sign. Sensation was abnormal in 25 patients (73.5%) and five (14.7%) had a sensory level. Three patients (8.8%) had optic atrophy. The cerebrospinal fluid showed a lymphocytic pleocytosis with a mean total protein content of 0.4 g/litre, and an increased intrathecal IgG synthesis in 59.4% of patients. HAM/TSP and multiple sclerosis (MS) occur indigenously in RJ and some HAM/TSP cases can be sometimes confused with MS. Therefore we propose that, in places where MS coexist with HAM/TSP, HTLV-I antibodies should be sought routinely in those MS suspected cases with prominent spastic paraparesis.
Adolescent↗
Tropical spastic paraparesis and human T cell lymphotropic virus type 1 in the United Kingdom.
Twenty-one patients with tropical spastic paraparesis (TSP), all of whom were born in the Caribbean and who had migrated to the United Kingdom, are described. All had a progressive spastic paraparesis developing many years after immigration and all 19 tested had antibodies to the human T cell lymphotropic retrovirus type 1 (HTLV1). The clinical and laboratory features and visual, auditory and somatosensory evoked potentials are described. Details of magnetic resonance scanning of the brain and, in a few cases, the spinal cord are compared with those found in multiple sclerosis. The antibody titres to HTLV1 assessed by particle agglutination, Western blot, antibody-directed cell mediated cytotoxicity and pseudotype neutralization were higher than in asymptomatic infected relatives and in patients with adult T cell leukaemia. Some, but not all, of the IgG oligoclonal bands in the CSF were directed against HTLV1. IgM oligoclonal bands directed against HTLV1 were found in 2 patients. Sixty of the 64 first degree relatives of 11 Jamaican patients with TSP were traced in the UK and the Caribbean; 20-30% of those born in the Caribbean had antibodies to HTLV1, irrespective of their present place of residence, whilst none of those born in the UK, who were the children of the patients, had antibodies. The original pathological material obtained from the Caribbean by Montgomery et al. (1964) is reviewed. These results are discussed in relation to animal retroviral neurological diseases, particularly visna in sheep which has clinical and pathological features closely similar to TSP. It is proposed that TSP is due to a HTLV1 infected lymphocyte/macrophage immune-mediated inflammatory response in the spinal cord.
Adult↗
[A case of late-onset tethered cord syndrome presenting with spastic paraparesis].
We report a patient with late-onset tethered cord syndrome who presented with slowly progressive spastic paraparesis. Final diagnosis was made with spinal MRI. A 55-year-old man developed disturbance in gait at 52 years. He had severe congenital scoliosis of the thoracic spine and skin anomaly and hypertrichosis in the midline of the sacral region. His feet revealed pes equinovarus and pes cavus. Deep tendon reflexes in the lower extremities were hyperactive with positive Babinski signs. Vibration sense was diminished in the lower extremities. He had sexual impotence but no vesical or rectal disturbances. The plain X-rays revealed scoliosis at the fourth and fifth thoracic vertebrae, multiple costal fusion from the fourth through the eighth ribs on the left, and spina bifida occulta at the lumbosacral vertebrae. On MRI, the conus was tethered downward at the level between the forth and fifth vertebral bodies, and a thickened filum terminale connected the conus with a coccygeal cyst. Tethered cord syndrome, though rare in adults, should be differentiated from other diseases which produce late-onset spastic paraparesis, and MRI of the lumbo-sacral region is very useful for its diagnosis.
Age of Onset↗
Brain atrophy in pure and complicated hereditary spastic paraparesis: a quantitative 3D MRI study.
Hereditary spastic paraparesis (HSP) is a heterogeneous group of neurodegenerative disorders with progressive lower limb spasticity, categorized into pure (p-HSP) and complicated forms (c-HSP). The purpose of this study was to evaluate if brain volumes in HSP were altered compared with a control population. Brain volumes were determined in patients suffering from HSP, including both p-HSP (n = 21) and c-HSP type (n = 12), and 30 age-matched healthy controls, using brain parenchymal fractions (BPF) calculated from 3D MRI data in an observer-independent procedure. In addition, the tissue segments of grey and white matter were analysed separately. In HSP patients, BPF were significantly reduced compared with controls both for the whole patient group (P < 0.001) and for both subgroups, indicating considerable brain atrophy. In contrast to controls who showed a decline of brain volumes with age, this physiological phenomenon was less pronounced in HSP. Therefore, global brain parenchyma reduction, involving both grey and white matter, seems to be a feature in both subtypes of HSP. Atrophy was more pronounced in c-HSP, consistent with the more severe phenotype including extramotor involvement. Thus, global brain atrophy, detected by MRI-based brain volume quantification, is a biological marker in HSP subtypes.
Adult↗
[Seroprevalence of HTLV-I in relatives of patients with spastic paraparesis (HAM/TSP and PEP)].
The seroprevalence of HTLV-1 was studied in parents, brothers, siblings and sexual partners of 147 patients with spastic paraparesis associated to HTLV-1 (HAM/TSP) and 84 patients with HTLV-1 negative spastic paraparesis (SP). Seroprevalence was 29.1% for HAM/SP and 0% for SP relatives (p < 0.001); the last figure is similar to that of the general population. Seroprevalence in sexual partners was 65%, suggesting that sexual intercourse is the principal route of transmission. Likewise, seroprevalence in siblings of mothers with HAM/TSP or HTLV-1 positive was 17.6%, suggesting a high maternal transmission.
Adolescent↗
Mu and delta, but not kappa, opioid agonists induce spastic paraparesis after a short period of spinal cord ischaemia in rats.
BACKGROUND: Intrathecal (IT) morphine given after a short interval of aortic occlusion in a rodent model induced transient spastic paraparesis via opioid receptor-predicted actions in spinal cord. To determine the role(s) of spinal opioid receptor subtypes we investigated whether IT administration of various selective opioid receptor agonists can induce paraparesis following a short period of spinal cord ischaemia in rats. METHODS: In Sprague-Dawley rats implanted with an IT catheter, spinal cord ischaemia was induced for 6 min using an intraaortic balloon. Mu ([D-Ala2, N-Me Phe4, Gly-ol5] enkephalin), kappa (U50488H) or delta ([D-Pen(2,5)] enkephalin) selective agonists were injected intrathecally 30 min after reperfusion. A separate group of animals was used to investigate the dose-response effect on this motor dysfunction. For this purpose, three doses of mu, kappa, or delta agonists were injected intrathecally after ischaemia. After IT injection, recovery of motor function was assessed periodically using the motor deficit index (0=complete recovery; 6=complete paraplegia). RESULTS: IT administration of mu and delta but not kappa agonists produced dose-dependent effects in the induction of spastic paraparesis. In addition, this spasticity induced by IT mu and delta agonists was reversed completely by IT naloxone and naltrindole, respectively. CONCLUSION: These results suggest that the effect of various opioids on motor function after a short period of spinal cord ischaemia depends upon individual opioid receptor subtypes.
3,4-Dichloro-N-methyl-N-(2-(1-pyrrolidinyl)-cycloh↗